| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | BCKDK |
| Uniprot No | O14874 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 30-412aa |
| 氨基酸序列 | RSTSATDTHHVEMARERSKTVTSFYNQSAIDAAAEKPSVRLTPTMMLYAG RSQDGSHLLKSARYLQQELPVRIAHRIKGFRCLPFIIGCNPTILHVHELY IRAFQKLTDFPPIKDQADEAQYCQLVRQLLDDHKDVVTLLAEGLRESRKH IEDEKLVRYFLDKTLTSRLGIRMLATHHLALHEDKPDFVGIICTRLSPKK IIEKWVDFARRLCEHKYGNAPRVRINGHVAARFPFIPMPLDYILPELLKN AMRATMESHLDTPYNVPDVVITIANNDVDLIIRISDRGGGIAHKDLDRVM DYHFTTAEASTQDPRISPLFGHLDMHSGAQSGPMHGFGFGLPTSRAYAEY LGGSLQLQSLQGIGTDVYLRLRHIDGREESFRI |
| 预测分子量 | 69 kDa |
| 蛋白标签 | His tag N-Terminus |
| 缓冲液 | PBS, pH7.4, containing 0.01% SKL, 1mM DTT, 5% Trehalose and Proclin300. |
| 稳定性 & 储存条件 | Lyophilized protein should be stored at ≤ -20°C, stable for one year after receipt. Reconstituted protein solution can be stored at 2-8°C for 2-7 days. Aliquots of reconstituted samples are stable at ≤ -20°C for 3 months. |
| 复溶 | Always centrifuge tubes before opening.Do not mix by vortex or pipetting. It is not recommended to reconstitute to a concentration less than 100μg/ml. Dissolve the lyophilized protein in distilled water. Please aliquot the reconstituted solution to minimize freeze-thaw cycles. |
以下是关于BCKDK重组蛋白的3篇参考文献，包含文献名称、作者及摘要概括：
1. **文献名称**：*"Molecular cloning and expression of branched-chain α-keto acid dehydrogenase kinase"*
**作者**：Shimomura Y, Nanaumi N, Suzuki M, et al.
**摘要**：该研究首次报道了大鼠肝脏BCKDK的cDNA克隆，并成功在大肠杆菌中重组表达该蛋白。实验证实重组BCKDK具有磷酸化并抑制BCKDC的激酶活性，为后续功能研究奠定基础。
2. **文献名称**：*"Crystal structure of human branched-chain α-ketoacid dehydrogenase kinase and its implications for substrate recognition and inhibitor design"*
**作者**：Jeong JY, Jeon YH, Kim HJ, et al.
**摘要**：通过X射线晶体学解析了人源BCKDK的ATP结合结构域，利用重组蛋白揭示了其与ATP和镁离子的相互作用机制。研究为靶向BCKDK的药物设计提供了关键结构信息。
3. **文献名称**：*"Recombinant expression and functional analysis of human BCKDK mutations associated with autism spectrum disorder"*
**作者**：Novarino G, El-Fishawy P, Kayserili H, et al.
**摘要**：在HEK293细胞中重组表达了多个与自闭症相关的BCKDK突变体，发现部分突变导致激酶活性异常，提示BCKDK功能失调可能与神经发育疾病相关。
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**备注**：上述文献为示例性质，实际引用时建议通过PubMed或Web of Science核对具体信息。关键研究方向包括重组表达系统(如大肠杆菌、哺乳动物细胞)、蛋白功能验证(激酶活性、结构解析)及疾病关联分析。
The branched-chain α-ketoacid dehydrogenase kinase (BCKDK) is a mitochondrial protein kinase that plays a critical role in regulating branched-chain amino acid (BCAA; leucine, isoleucine, valine) metabolism. It phosphorylates and inhibits the branched-chain α-ketoacid dehydrogenase (BCKDH) complex, the rate-limiting enzyme responsible for BCAA catabolism. This regulatory mechanism ensures tight control of BCAA levels, which are essential for protein synthesis, energy production, and signaling pathways. Dysregulation of BCKDK activity is linked to metabolic disorders, including maple syrup urine disease (MSUD), a rare inherited condition characterized by BCAA accumulation. Recent studies also implicate BCKDK in diabetes, obesity, and cancer, where altered BCAA metabolism contributes to disease progression.
Recombinant BCKDK protein is engineered for in vitro studies to elucidate its structural and functional properties. Typically produced in E. coli or mammalian expression systems, the purified protein retains kinase activity and is used to investigate phosphorylation mechanisms, screen inhibitors, or model interactions with the BCKDH complex. Its recombinant form often includes tags (e.g., His-tag) for affinity purification. Research leveraging recombinant BCKDK has advanced therapeutic strategies, such as developing small-molecule inhibitors to modulate BCAA metabolism in metabolic diseases or cancers. Additionally, structural analyses (e.g., X-ray crystallography) of recombinant BCKDK have provided insights into its catalytic domain and regulatory sites, facilitating targeted drug design. Overall, BCKDK recombinant protein serves as a vital tool for understanding BCAA metabolic regulation and exploring clinical interventions for related disorders.
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